The journey of more than 6 months to save a patient with a rare disease was reported for the first time in Vietnam.

On the afternoon of June 27, Bach Mai Hospital held a farewell ceremony for patient Nguyen Quoc Trinh after nearly 200 days of treatment for an extremely rare disease. This is the first case recorded in Vietnam.

In December 2023, the patient was admitted to the Department of Dermatology (Bach Mai Hospital) with severe oral mucosal ulcers, accompanied by skin lesions with manifestations of papules, purple-red patches, blisters, and vesicles scattered all over the body. The skin lesions quickly progressed into large skin erosions, accounting for up to 70% of the body surface area. The oral mucosal ulcers were very painful, causing the patient to have a poor appetite and quickly become exhausted.

In addition to serious skin and mucosal manifestations, doctors also discovered that the patient had a large retroperitoneal tumor, measuring about 136x65x131mm, pressing on many surrounding organs.

With diverse skin lesions, the patient was indicated for skin biopsies many times, but the biopsies gave different results.

Associate Professor, Dr. Dao Xuan Co handed discharge papers to patient Nguyen Quoc Trinh on the afternoon of June 27.

Faced with the extremely rapid progression of skin lesions, with a high risk of affecting life, Bach Mai Hospital has organized many hospital-wide consultation councils with the participation of many leading experts... However, the clinical signs and complex test results, each time giving a different result, have made it impossible for the Bach Mai Hospital consultation council to come to a final conclusion for a suitable treatment plan.

Not giving up, the doctors searched through medical literature, found similar cases and came up with the final diagnosis. Patient Nguyen Quoc Trinh was diagnosed with Pemphigus paraneoplastic disease with evidence of indirect immunofluorescence testing on mouse bladder showing positive IgG results in the intercellular space and basement membrane.

Specialist II Tran Thai Son, Head of Dermatology Department, said: Paraneoplastic pemphigus is a rare autoimmune skin disease, related to tumors in the body. Tumors produce abnormal proteins and are the target of the body's immune system, creating cross-immunity with proteins of the skin and mucous membranes. The most common are malignant lymphoproliferative or hematological tumors.

This is a very rare disease in dermatology and there are very few data in the world to allow an estimate of its incidence. Based on published reports, the estimated incidence of pemphigus is less than 1/1 million population per year.

Image of patient's skin lesions.

In Vietnam, data on paraneoplastic pemphigus is still quite limited and no case reports have been published.

The diagnosis of Pemphigus vulgaris has been difficult, but the treatment of the disease is an extremely big challenge for doctors, requiring the coordination of many specialties and leading experts. With the diagnosis of Pemphigus vulgaris , tumor treatment is one of the first steps to treat the disease. However, due to the extensive skin lesions and exhausted physical condition, the patient was not healthy enough to undergo a major surgery. The hospital's consultation board agreed to treat the skin lesions and improve the patient's physical condition before surgery.

In the early stages, the patient's lesions responded poorly to conventional immunosuppressive therapy. The skin lesions continued to spread to more than 80% of the body surface, with severe damage to the mucous membranes of the eyes, ears, and mouth. The patient rapidly lost 10 kg within 1 month.

There were times when the patient and his family gave up and expressed their wish to go home. But the doctors refused to give up. With the determination of the white-coat soldiers and the family's solidarity, Trinh returned to life.

Representatives of Hanoi Social Insurance congratulated the patient on being discharged from the hospital.

When the skin lesions had healed >95%, the oral mucosa lesions had partially recovered, the patient quickly fell into a new outbreak with a more severe and complicated course than the previous one. Once again, the doctors and nurses raised their determination, trying their best to save the patient's life. After 3 months of treatment, with the tireless efforts of the doctors and the patient's family, the skin lesions had recovered, the patient's health had improved, and the patient was eligible for surgery to remove the tumor.

The surgery fortunately went smoothly despite the extremely difficult prognosis. Post-operatively, the patient was stable, skin and mucosal lesions recovered. No new skin lesions appeared, oral mucosal lesions improved. The patient was able to eat and drink. The dose of immunosuppressant drugs was gradually reduced. And on June 25, the consultation board concluded that he was fit to be discharged from the hospital.

Source: https://nhandan.vn/hanh-trinh-hon-6-thang-cuu-benh-nhan-mac-benh-hiem-lan-dau-duoc-bao-cao-tai-viet-nam-post816463.html